Search

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, presenting either in isolation or with other cardiac anomalies. The most common presentation is congestive heart failure in infancy, though some may remain asymptomatic till adulthood. A 5-month-old infant with congestive heart failure was diagnosed to have ALCAPA with severe Left ventricular (LV) dysfunction. Unlike the more common variants where the left coronary artery (LCA) arose from the facing sinuses, the LCA arose from the left anterior nonfacing sinus of the pulmonary artery at a considerable distance from the left aortic sinus. This made direct reimplantation of LCA to the aorta challenging. LCA was reimplanted to the aorta using an extrapulmonary baffle made of an autologous pulmonary flap. The postoperative period was uneventful. The LV contractility improved during the follow-up. Early diagnosis and intervention with a novel surgical technique prevented irreversible cardiac complications and mortality.

Transcatheter closure of sinus venosus defect (SVD) is an emerging intervention that utilizes a covered stent to redirect the right upper pulmonary vein to the left atrium. Preprocedural computed tomography analysis, as well as the interventional procedure, necessitates the use of radiographic contrast media. Contrast use is prohibited in patients with advanced kidney disease, who also carry high surgical risks of cardiopulmonary bypass. Transesophageal echocardiography-guided catheter intervention with zero contrast use is presented in this report, along with technical details about planning the procedure. Covered stent exclusion of SVD without contrast use has not been reported in the literature so far.

Transcatheter closure of superior vena cava (SVC) form of sinus venosus defects (SVDs) using covered stents is emerging as an alternative to surgery in the current decade. A covered stent placed in the cavoatrial junction creates a roof for the right upper pulmonary vein (RUPV) that stops the left-to-right shunt and redirects the vein to the left atrium. While surgical literature has clearly documented the incidence of stenosis of SVC and RUPV, sinus nodal dysfunction, and persistent residual shunts following surgical correction, it is imperative to have similar data after this new transcatheter intervention on the incidence of complications and follow-up outcomes. Since patients with pretricuspid shunts are often clinically asymptomatic, correction is primarily performed to prevent a persistent right heart volume overload and allow remodeling of the heart chambers. Any residual left-to-right shunt after a correction will result in persistent right heart dilatation. Residual flows can result from various mechanisms, including lack of apposition of the covered stent to the free edge of the SVD, fabric breach, and persistent anomalous drainage of additional right-sided pulmonary veins that drain very high in the SVC or can be due to a coexistent defect in the oval fossa. This review analyzes the different mechanisms, explains the transesophageal and angiographic images for each one, and offers solutions tailored for various reasons. Different mechanisms warrant different treatment principles. A solution for residual shunt from one mechanism may not be appropriate for residual flow through another mechanism. A thorough understanding would aid the operator in effective interventions for these SVDs.

Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients. Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis. Results: Among 21 patients including 8 males with a median age of 3 years (18 days–72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing

Transcatheter sinus venosus defect (SVD) closure with covered stents is emerging as an alternative to surgery. An adequate anchor zone in the superior vena cava is mandatory for the stability of the covered stent to prevent caudal embolization. There is a potential risk of innominate vein occlusion by the fabric of the covered stent in patients with a very short superior caval vein. Three among a total of 105 patients who underwent SVD closure at our institution developed innominate vein occlusion. Predisposing anatomical factors, identification and management of occluded innominate vein, and follow-up outcomes are discussed.

Atrial septal defects (ASDs) measuring

Background : Junctional ectopic tachycardia (JET) is the most common arrhythmia after pediatric open-heart surgeries (OHS), causing high morbidity and mortality. As diagnosis is often missed in patients with minimal hemodynamic instability, its incidence depends on active surveillance. A prospective randomized trial evaluated the efficacy and safety of prophylactic amiodarone and dexmedetomidine to prevent and control postoperative JET. Methods : Consecutive patients aged under 12 years were randomized into amiodarone, dexmedetomidine (initiated during anesthetic induction) and control groups. Outcome measures included incidence of JET, inotropic score, ventilation, and intensive care unit (ICU) duration and hospital stay, as well as adverse drug effects. Results : Two hundred and twenty-five consecutive patients with a median age of 9 months (range 2 days–144 months) and a median weight of 6.3 kg (range 1.8 kg–38 kg) were randomized with 70 patients each to amiodarone and dexmedetomidine groups, and the rest were controls. Ventricular septal defect and Fallot's tetralogy were the common defects. The overall incidence of JET was 16.4%. Syndromic patients, hypokalemia, hypomagnesemia, longer bypass, and cross-clamp duration were the risk factors for JET. Patients with JET had significantly prolonged ventilation (P = 0.043), longer ICU (P = 0.004), and hospital stay (P = 0.034) than those without JET. JET was less frequent in amiodarone (8.5%) and dexmedetomidine (14.2%) groups compared to controls (24.7%) (P = 0.022). Patients receiving amiodarone and dexmedetomidine had significantly lower inotropic requirements, lower ventilation duration (P = 0.008), ICU (P = 0.006), and hospital stay (P = 0.05). Adverse effects such as bradycardia and hypotension after amiodarone and ventricular dysfunction after dexmedetomidine were not significantly different from controls. Conclusion : Prophylactic amiodarone or dexmedetomidine started before OHS is effective and safe for the prevention of postoperative JET.

Covered stent is used in large-vessel angioplasty in anticipation of vessel wall injury. Apart from aortic coarctation, they are also used in dysfunctional right ventricular outflow conduits and find a recent role in transcatheter sinus venosus defect closure. Different methods of covering stents include glue fixation, sutureless lamination, sandwich, and sintering lamination. Covered Zephyr (Sahajanand laser technology limited, Gandhinagar, India) is a new Indian-made expanded polytetrafluoroethylene-covered balloon expandable cobalt-chromium stent. Its unique C and S links prevent foreshortening. We report the first-in-man use of this new stent in severe discrete postsubclavian coarctation of aorta and its short-term follow-up imaging.

Transcatheter correction of superior sinus venosus defects using covered stent is increasingly reported in the literature and provides an alternative option to surgery in appropriately selected adults. Being a new intervention, meticulous attention to procedural techniques and precise surveillance imaging modalities are vital to detect and avoid potential early and late complications. This report highlights the occurrence of a residual interatrial communication following covered stent placement and large subclinical asymptomatic nonocclusive thrombus formation at the right atrial end of the stent. The management of both these complications is also highlighted in this report.

Anomalous left coronary artery origin from pulmonary artery causes heart failure in infancy from ischemia and secondary mitral regurgitation. Rich intramyocardial collateralization may permit survival to adult age, where coronaries become tortuous and aneurysmally dilated. Surgery in adults involves left coronary ligation and providing a bypass graft to the left system, unlike coronary translocation adopted in infants. Unfavorable coronary remodeling in operated adults may lead to late coronary thrombotic occlusions. Two adults with markedly dilated tortuous coronary arteries showed variable remodeling after corrective intervention that impacted outcomes on follow-up. We stress the need for lifelong angiographic surveillance in older patients.

Background: Right ventricular (RV) dysfunction is an important predictor of adverse events after tetralogy of Fallot (TOF) repair. A study comparing echocardiography with cardiac magnetic resonance (CMR) might provide simple tools for their serial inexpensive assessment. Methods: Echocardiographic RV functional parameters including fractional area change (FAC), rate of pressure rise (dP/dt), free wall strain, myocardial performance index (MPI), tricuspid annular plane systolic excursion (TAPSE), and tissue Doppler S' velocity in patients with repaired TOF were correlated with CMR-derived ejection fraction (EF) using receiver operator characteristic (ROC) curves. Bland–Altman plots analyzed agreement between the RV end-diastolic volumes (EDVs), end-systolic volumes (ESVs), and EF derived from three-dimensional echocardiography (3DE) and CMR. Results: Eighteen patients were included. The echocardiographic parameters that showed a good positive correlation with CMR-derived EF were FAC (r = 0.851, P = 0.001), dP/dt (r = 0.730, P = 0.001), and free wall strain (r = −0.660, P = 0.003). ROC curve analysis provided a cutoff value for FAC (0.565) to predict EF

Segmental pulmonary arterial hypertension occurs when the right and left pulmonary arteries are nonconfluent and receive blood supply from different sources. High blood pressure confined to limited lung segments may accelerate progression of pulmonary vascular resistance. Calculation of segmental vascular resistance and assessment of operability in such situations are done after integrating catheter hemodynamics, magnetic resonance imaging techniques, or perfusion scintigraphy. When an isolated pulmonary artery perfused by a restrictive ipsilateral arterial duct is associated with a large nonrestrictive contralateral arterial duct connected to the other pulmonary artery, leading to unilateral pulmonary arterial hypertension and features of high vascular resistance, it offers unique challenges to decision-making.

Superior sinus venosus defect is characterized by varying degrees of override of the atrial septum by the superior vena cava and anomalous right upper pulmonary vein (RUPV) drainage. The defect is seen posterosuperior to the oval fossa and is traditionally corrected by a surgical patch that routes the anomalously draining RUPV to the left atrium. A few postoperative patients develop stenosis or occlusion of the caval or pulmonary vein and/or sick sinus syndrome secondary to vascular injury. This has kindled a recent interest in nonsurgical correction of these defects by the use of a covered stent to reroof the defect. The complex anatomy of this defect often necessitates the use of advanced imaging tools and postprocessing. Countries with restricted resources do not have easy access to technologies such as three-dimensional printed models and advanced processing like virtual reality or holography. This “How to do it?” article, applicable in these countries, explains the current understanding of this intervention, patient selection, step-by-step explanation of the evaluation, and stent implantation.

Coronary arteries coursing behind the aortic root may get compressed when nitinol septal occluders are used to close an atrial septal defect. Hence, echocardiographic recognition of a retroaortic linear vessel is important during preinterventional evaluation. While the left circumflex arising from the right coronary artery is the most common cause, a similar finding is sometimes observed in a single left or right coronary artery and rarely with small sinus nodal branches from the left circumflex artery. Complex three-dimensional relations between the defect and the aortic root may be understood only after a postdeployment selective coronary angiography. Two patients with anomalous retroaortic left circumflex from the right coronary artery underwent uneventful device closure with clearly documented separation between the edges of the occluder and the anomalous vessel. Follow-up imaging and exercise testing confirmed the safety of the intervention. A selective postdeployment and postrelease coronary angiography are mandatory in every patient with retroaortic coronaries.

Background: Device closure of multiple atrial septal defects (MASDs) is frequently done using a single centrally deployed septal or cribriform occluder, but multiple devices are needed for large defects separated more than 6 mm. There is a concern about complications while using multiple devices, especially in children. Methods: Patients who received multiple devices for closure of MASD were grouped according to their age and analyzed for procedural techniques, immediate and late complications. MASDs closed by a single device were not included. Balloon sizing was done when echocardiographic images were suboptimal before simultaneous device deployment through two venous accesses or sequential deployment through one access. Duration and number of antiplatelet drugs and residual flows were analyzed on follow-up. Results: Twenty-five patients received multiple devices. Balloon interrogation was performed in 16/18 adults but only in 2/7 children. Device size was 2–5 mm larger than echocardiographic defect size or equal to balloon waist. There were no procedural failures; 7/25 showed small postprocedural residual flows. Complications including embolization in one, arrhythmia in one, and cobra deformity in two were managed successfully. On a median follow-up of 5.5 years (1–12 years), residual flows disappeared in 4/7 and there were no major late complications. Conclusions: Use of multiple devices for closing MASD is feasible with good technical success. Echocardiography and balloon interrogation are the keys for success. Simultaneous deployment is often needed and sequential delivery is feasible rarely if the defects are far apart. Minor residual leaks are common but improve on follow-up. There are no significant new complications on long-term follow-up.

Dysfunctional right ventricular outflow tracts after a repair for tetralogy of Fallot using a transannular patch offer limited nonsurgical opportunities due to their large dimensions. A discrete subannular narrowing between a dilated right ventricle and the enlarged pulmonary trunk was a potential anatomical target for the creation of a landing zone using a prestent in a young male with severe pulmonary regurgitation and moderate stenosis. Asymmetric expansion of the prestent in the angulated outflow tract led to distal stent embolization that was stabilized by another telescoping stent before successful valve implantation. This manuscript details the unique challenges posed by a dilated regurgitant outflow tract for implanting a pulmonary valve.

Acute ruptured sinus of Valsalva aneurysm may present with chest pain and rapidly worsening dyspnea. A 41-year-old male without known atherosclerotic risk factors presented with ST-segment elevation in leads aVR and V1 on electrocardiogram and elevated cardiac troponins and was diagnosed as acute coronary syndrome. After antiplatelet loading, statin, and heparin, he was referred due to worsening hypotension and tachycardia. Clinical examination and echocardiogram was challenging in the acutely dyspneic restless patient. Transesophageal echocardiography delineated the ruptured aneurysm and assisted in its management. Importance of physical examination and echocardiography in the emergency room is highlighted as manifestations may be varied.

Extreme pulmonary artery hypoplasia in cyanotic malformations precludes palliative surgeries. When aortopulmonary collaterals (APC) in such patients are also hypoplastic, their unifocalization to create a neopulmonary vasculature is also hampered. Stent angioplasty of the outflow or collateral arteries may reduce hypoxia but is challenging in tortuous and atretic tracts. Fusion imaging overlays anatomical data from computed tomography during adult structural interventions, but its use is not often reported in young children with complex cyanotic malformations. This report shows utility of fusion imaging in pulmonary atresia with extremely hypoplastic pulmonary arteries and stenotic APC to guide stenting of outflow tract and collaterals.

Pulmonary arteriovenous malformations after the Kawashima procedure causing severe hypoxemia are treated by Fontan surgery that redirects hepatic venous blood to the pulmonary circulation. Alignment of the hepatic venous conduit toward both pulmonary arteries is crucial for their regression. Persistent hypoxemia due to nonregressed malformations in one lung often warrants a repeat surgery to redirect hepatic venous flows. Catheter-based redirection of Fontan flows is not commonly performed as metallic stents and devices may predispose to thrombus formation in the circuit. This report highlights a patient with persistent arteriovenous malformations in the left lung leading to severe disabling hypoxemia that was managed in the catheterization laboratory with an innovative strategy to redirect Fontan flows toward the affected left lung.

Splenic artery aneurysms (SAAs) are the third most frequent site of intraabdominal aneurysm and the most frequent splanchnic artery aneurysm. Surgery may be morbid and may include splenectomy and distal pancreatectomy. True aneurysm needs differentiation from a pseudoaneurysm caused by pancreatic enzymatic destruction. A giant SAA was diagnosed in an elderly atherosclerotic male with chronic calcific pancreatitis. Layered thrombus on multimodal imaging ruled out enzyme action and excluded possibility of a pseudoaneurysm. The distal splenic artery beyond the aneurysm was collateralized by left gastric artery. The aneurysm was closed with multiple coils and a vascular plug. Imaging after 6 months confirmed complete closure. Endovascular closure of SAA needs strategies to avoid an endoleak from the distal splenic artery. Pseudoaneurysm of splenic artery should be excluded, as they do not respond to the same strategies applied for true atherosclerotic aneurysms.

Amplatzer Paravalvular leak (PVL) plug is rectangular in shape, that might suit closure of crescentic PVL. Among 79 transcatheter PVL closures from a single center, a subgroup of 16 patients who received Amplatzer PVL plugs were analyzed. All procedures were successful, as the plug auto-oriented to the leak, without mechanical leaflet interference, though needing additional 31 devices. Two patients needed an elective re-intervention. NYHA class improved from III-IV before procedure to less than II after procedure. Complications were not directly related to the Amplatzer PVL plug. The rectangular shape for crescentic leaks, auto-orientation and non-interference with mechanical leaflets were good attributes.

Cardiac tamponade in children has multiple etiological factors such as infection, post-inflammatory, malignancy, or blunt trauma. Detailed evaluation of the accompanying clinical features along with laboratory investigations and imaging help in appropriate diagnosis and management. We describe a case where cardiac tamponade manifested as the first presentation of childhood lymphoma but was mistaken as post-traumatic hemopericardium and tamponade.

Background : Outcome data of children with heart disease who acquired COVID-19 infection are limited. Aims : We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. Settings and Design : This is a retrospective, multicentric, observational study. Materials and Methods : The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. Results : From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3–96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9–41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1–814.7, P = 0.046). Conclusions : Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

Hemodynamically significant large pulmonary embolism causes right ventricular dysfunction that stretches open the foramen ovale in the atrial septum. Paradoxical embolism is common in patients with right ventricular dysfunction. While anticoagulation and thrombolysis are considered the treatment of choice in pulmonary embolism, a large thrombus in transit across the patent foramen ovale carries a serious risk of systemic thromboembolism and warrants urgent surgery. The recent coronavirus disease 2019 (COVID-19) pandemic has led to a significant increase in the incidence of thromboembolic illnesses. A patient acutely presented with deep vein thrombosis, pulmonary embolism, large thrombus in transit across the foramen ovale along with elevation of multiple inflammatory biomarkers that were suggestive of a possible post-COVID sequelae. Images and management strategies are discussed in this report.

Background : Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). Methods : Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. Results : Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure (n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1–39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. Conclusions : Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients.

Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. Methods : Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1–4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. Objectives : A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. Results : Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1–85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. Conclusion : Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.

Background : Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI. Methods : Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed. Results : Seven patients aged 17–60 years (median 26 years) had stenosed RV-PA conduits implanted 5–17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50–137 mmHg) to 12.7 ± 6.4 mmHg (5–20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10–22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events. Conclusions : Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Background : COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. Aims : The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design : This is a retrospective, multicentric, observational study. Methods : We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions : The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Objectives: Left atrial appendage occlusion (LAAO) in non-valvar atrial fibrillation (AF) reduces cardioembolic strokes. Despite increased risk, trials exclude valvar AF in structural heart diseases where clots extend beyond appendage. Methods: Patients with AF and relative risks for oral anticoagulation (OAC) needing structural interventions underwent concomitant LAAO. After six months of OAC, aspirin was continued. Transesophageal echocardiogram was done three monthly till one year and yearly thereafter. The patient demographics, procedural details, post-procedural follow-up were analyzed. Results: Nine patients aged 51.5 ± 6.3 years with AF underwent LAAO concomitantly with balloon mitral valvotomy in four patients, atrial septal defect device closure in four and periprosthetic mitral leak closure in one patient. Six patients had heart failure, four had prior embolic events, and two had documented LAA thrombus. The mean CHADS2VASc score was 2.44 ± 0.8 and mean HASBLED score was 3.0 ± 0.8. Devices included Amplatzer Cardiac Plug™ in six patients, LAmbre™ Lifetech device in two and Watchman™ device in one. All procedures were successful without acute complications. A patient developed pericardial effusion at six months requiring pericardiocentesis. Early device-associated thrombus in one patient resolved after OAC for six months. No embolic events occurred on follow-up. Conclusion: On a detailed literature search, this largest LAAO experience in structural heart diseases indicates its utility. OAC for six months followed by aspirin seems to prevent thrombus formation in these patients. The only incidence of early thrombus formation indicates immunity from clot formation after device endothelialisation. Larger multicenter trials combining LAAO with structural interventions in valvular AF are warranted in developing nations.

Spontaneous aortic thrombosis is a serious clinical entity with clinical features that mimic neonatal coarctation of aorta. While both conditions manifest with weak femoral pulses, pallor and poikilothermia of lower limbs are unique to spontaneous aortic thrombosis. Proper imaging helps in precise diagnosis. A combination of thrombolysis and anticoagulation is used for resolution of thrombus. We present images of a neonate with spontaneous aortic thrombosis and its clinical management strategies.

Mycotic aneurysms of pulmonary arteries are potentially fatal conditions occurring in patients ith congenital heart disease complicated by infective endocarditis folloing surgery or interventions. Presentation is indolent and symptoms may be nonspecific. Unless suspected and investigated ith computed tomography, they are missed until their advanced stage. Treatment options are limited as shon in to patients presented in this report.

Double-outlet right atrium (DORA) is a rare clinical entity associated with atrioventricular septal defects. Although it is easily correctable by surgery, it may be misdiagnosed during echocardiographic evaluation. Awareness and a thorough knowledge of this entity, its associations, echocardiographic appearances, surgical techniques, and its embryogenesis can alert the clinician to identify this rare but correctable condition. A young patient with DORA is described in this clinical report.

Fontan surgery streamlines the systemic venous return through the pulmonary circulation before filling the systemic ventricle in univentricular hearts. The venous congestion leads to effusions, lowers cardiac output, and affects organ perfusion. Fenestrations in the Fontan circuit improve forward flow through the ventricles, lower venous pressures, and reduce perioperative morbidity. When large fenestrations cause profound hypoxia and effort intolerance, there are no current techniques to reduce their size. Atrial flow regulators with a predetermined orifice were used off-label in three borderline patients with large undesirable fenestrations following extracardiac conduit Fontan surgeries. This resulted in improved oxygenation without marked elevation of venous pressures, while retaining the patency of the decompressive fenestration.

Double outlet ventricle is a type of conoventricular anomaly. The great arteries arise predominantly (more than 50%) from one of the ventricle. The aortomitral discontinuity varies from minimum discontinuity to complete bilateral conal tissue. Both right and left ventricles can have double outlet though double outlet right ventricle (DORV) is the most common anomaly in this group of abnormalities. Double outlet left ventricle (DOLV) is relatively rare. There are multiple types of DORV based on the site of ventricular septal defect and great artery relationship and presence or absence of ventricular outflow obstruction. The correct anatomical diagnosis requires a detail segmental approach in echocardiography. The echocardiographic features of DORV reviewed in detail followed by a brief discussion on DOLV.

Coronary allograft vasculopathy fails to give a warning anginal pain due to denervation and often presents with acute coronary syndrome, ventricular dysfunction, or sudden cardiac death. Early diagnosis in a pediatric patient is difficult as it involves invasive coronary angiography or advanced imaging such as intravascular ultrasound or optical coherence tomography. A 12-year-old boy developed acute coronary syndrome, elevated troponins, and right bundle branch block, 5 years after cardiac transplantation and was treated with culprit-vessel angioplasty with a drug-eluting stent. Advanced imaging showed the involvement of nonculprit vessels too. In a detailed literature search, we failed to identify a similar clinical presentation and management in the subcontinent, hence our interest in publishing this report for educational value. Issues in diagnosis, management, prognosis, and prevention are discussed.

Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension. Occlutech Atrial Flow Regulator is designed to provide an atrial septal fenestration diameter titrated according to the age and right atrial pressures. This observational study analyzed symptoms, exercise distance, oxygen saturations, hemodynamics and echocardiographic parameters after Atrial Flow Regulator implantation in patients with syncope or right-heart failure. Patients with high-risk predictors of mortality during septostomy were scrutinized. Thirty-nine patients (9 children) with syncope (34/39) or right-heart failure (27/39) underwent Atrial Flow Regulator implantation without procedural complications. Six-minute walk distance increased from 310 ± 158.2 to 376.4 ± 182.6 m, none developed syncope. Oxygen saturations reduced from 96.4 ± 6.4% to 92 ± 4.9% at rest and further to 80.3 ± 5.9% on exercise. Right atrial pressures reduced from 9.4 ± 5 (2–27) mmHg to 6.9 ± 2.6 (1–12) mmHg, while cardiac index increased from 2.4 ± 0.8 (0.98–4.3) to 3 ± 1 (1.1–5.3) L/min/m 2 and systemic oxygen transport increased from 546.1 ± 157.9 (256.2–910.5) to 637.2 ± 191.1 (301.3–1020.2) ml/min. Echocardiographic improvement included significant reduction of pericardial effusion and inferior caval congestion at a median follow-up of 37 months. Overall survival improved except two early and one late deaths in high-risk patients. Five of seven patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in pulmonary arterial hypertension after Atrial Flow Regulator were improved cardiac output, systemic oxygen transport, and reduced right atrial pressures. Improvement of symptoms especially syncope, exercise duration, and right ventricular systolic function as well as device patency were sustained on mid-term follow-up. Implantation was safe in all including young children without procedural complications. Mortality was noted only in patients who had high-risk predictors and patients at advanced stage of the disease.

Fontan surgery and its modifications have improved survival in various forms of univentricular hearts. A regular atrial rhythm with atrioventricular synchrony is one of the most important prerequisite for the long-term effective functioning of this preload dependent circulation. A significant proportion of these survivors need various forms of pacing for bradyarrhythmias, often due to sinus nodal dysfunction and sometimes due to atrioventricular nodal block. The diversion of the venous flows away from the cardiac chambers following this surgery takes away the simpler endocardial pacing options through the superior vena cava. The added risks of thromboembolism associated with endocardial leads in systemic ventricles have made epicardial pacing as the procedure of choice. However challenges in epicardial pacing include surgical adhesions, increased pacing thresholds leading to early battery depletion and frequent lead fractures. When epicardial pacing fails, endocardial lead placement is equally challenging due to lack of access to the cardiac chambers in Fontan circulation. This review discusses the univentricular heart morphologies that may warrant pacing, issues about epicardial pacing, different techniques for endocardial pacing in patients with disconnected superior vena cava, pacing in different modifications of Fontan surgeries, issues of systemic thromboembolism with endocardial leads, atrioventricular valve regurgitation attributed to pacing leads and device infections. In a vast majority of patients following Glenn shunt and Senning surgery, an epicardial pacing and lead replacement is always feasible though technically very difficult. This article highlights the different options of transatrial and transventricular endocardial pacing. Keywords: Fontan surgery, Bradyarrhythmia, Transvenous pacing, Epicardial pacing, Univentricular hearts, Systemic thromboembolism

Scimitar syndrome is a clinical triad of anomalous pulmonary venous drainage, lung hypoplasia, and anomalous aortic blood supply to the lung segment. When there is dual pulmonary venous drainage both to inferior vena cava and left atrium, it is called scimitar variant. A young child presenting with recurrent chest infections, dextroposition of the heart, and scimitar shadow on chest X-ray was identified to have scimitar variant after a detailed evaluation and managed successfully by catheter interventions. This report discusses the embryogenesis and angiographic findings of scimitar variants, indications for interventions, and issues in its management.

Objectives: Narrowed right ventricular (RV) outflow conduits and pulmonary arteries (PA) increase RV pressures and warrant interventions. Stent angioplasty is an alternative to more morbid redo-surgery in developing countries. We evaluate the efficacy and safety of stenting and assess need for redo-surgical reinterventions on midterm follow-up after stent angioplasty. Methods: Patients who underwent conduit, main PA and bilateral branch PA stenting for elevated RV pressures were analyzed retrospectively. Success was defined as 20% reduction in RV pressures or RV-aortic pressure ratio; 50% reduction in gradients or 50% increase of luminal diameter. Procedural results, complications and need for redo surgeries on follow-up were assessed. Results: Among 60 patients aged 1–46 years, 57 were post-operative patients, who needed stenting at a median period of 48 months after surgery. Stenting succeeded in 98% and reduced RV pressures from 105.42 ± 28.39 mmHg to 54.46 ± 16.89 mmHg. Direct major procedural complications in five (8%) patients included procedural failure in one, stent migration in three and lung hemorrhage in one. None of the stented conduits needed a surgical change on a follow-up ranging 3–120 months. Following bilateral PA stenting in twenty-four patients, only two needed a repeat open-heart surgery during follow-up ranging 3–108 months. Catheter reinterventions on follow-up included elective percutaneous pulmonary valve implantation in nine patients and stent redilation in seven patients. Conclusions: Stent angioplasty was safe and effective. Surgery was postponed in all stenosed conduits. Elective redilation of stents after bilateral PA stenting may be needed for somatic growth; but open-heart repeat surgeries can be avoided in a majority. Keywords: Stent angioplasty, Conduit stenting, Pulmonary artery stents, Bifurcation stenting

Right ventricular (RV) geometry is altered by septal shift after pulmonary artery banding. This may reduce tricuspid regurgitation (TR) and improve ventricular function in patients with corrected transposition of great arteries and systemic right ventricle. However, banding is risky in sick patients with severe RV failure. There are no predictive models in clinical practice to test this septal shift hypothesis before a risky surgery. A transcatheter model to mimic a pulmonary artery band is presented in corrected transposition of great arteries with failing right ventricle and severe TR.

PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphological differences. Corkscrew aortic arch, an extreme tortuosity of the aortic arch described in arterial tortuosity syndrome, is not reported in PHACES syndrome so far. Multimodal imaging of this unusual corkscrew aortic arch in two patients with PHACES syndrome is presented.

Duplication of atrioventricular valves involves the mitral valve more often than the tricuspid valve and is often associated with other cardiac defects. Double-orifice tricuspid valve (DOTV) is often identified in surgery or autopsy and missed on echocardiography, as the two orifices are orthogonal to the imaging plane. If suspected on echocardiography, it masquerades as mild tricuspid hypoplasia. Three-dimensional echocardiography and magnetic resonance imaging of a DOTV are presented.

Complex cyanotic congenital heart diseases with left isomerism are sometimes associated with atrioventricular nodal conduction disturbances that may need permanent pacing. Surgical palliation in such anatomy connecting the superior vena cava to the pulmonary artery precludes a transvenous access for an endocardial pacing lead to the ventricles. Epicardial leads in these patients fail if the pacing thresholds are very high. We report transhepatic permanent ventricular lead implantation for a young boy with heterotaxy complicated by complete heart block.

Double aortic arch resulting from embryonic persistence of bilateral fourth branchial arches often leads to neonatal stridor due to tracheal compression. Two neonates presented with dyspnea without evidence of tracheal compression. An aneurysmal ductal aneurysm from the left-sided arch in the first neonate led to symptoms. Spontaneous duct closure and regression of ductal aneurysm led to relief of symptoms. Second neonate had severe neonatal pulmonary hypertension due to aortic origin of left pulmonary artery (LPA) that was disconnected from main PA. Surgical reanastomosis of the LPA and division of the smaller right arch corrected the anatomy. These two associations show that airway obstruction alone is not the cause of respiratory symptoms in neonates with double aortic arch.

Coronary sinus defects refer to interatrial communications that lie out of the confines of the atrial septum and leads from left to right shunt through the ostium of the coronary sinus. When associated with persistent left superior vena cava (PLSVC), mild systemic desaturation may occur depending on the extent of unroofing of the coronary sinus. Isolated defects without PLSVC are rare. Three-dimensional echocardiographic and surgical images are presented.

Test occlusion with a balloon is done to predict operability of large hypertensive patent ductus arteriosus (PDA). If the fall in the pulmonary artery pressures is inadequate, a complete closure is not desired. To create a predictable premeasured fenestration in a nitinol occluder device used for closing hypertensive PDA. A large nitinol occluder device was punctured with an 18G needle to advance a 0.035½ stiff guide wire through the occluder before loading it into the delivery system. The occluder with the guidewire was then deployed across the PDA. A coronary guide catheter was later threaded through the guidewire into the fabric of the device, which was still held by the delivery cable. A coronary stent was deployed across the fenestration in the occluder to keep it patent. An 8-year-old boy with Down syndrome and hypertensive PDA was hemodynamically assessed. Even though there was a fall in the pulmonary vascular resistance index and pressures on test occlusion, the pulmonary artery pressures were labile with fluctuations. A customized fenestration was made in a 16 mm muscular ventricular septal defect occluder (MVSO) with a 4.5 mm bare-metal coronary stent. The pulmonary artery pressures remained at half of the aortic pressures after the procedure. This fenestration model precisely and predictably fenestrated a large occluder device used to close a hypertensive large PDA. Long-term patency of these fenestrations has to be assessed on the follow-up, and may be improved through larger fenestrations, systemic anticoagulation and use of covered stents.

Background : Embolization might complicate device closure of large atrial septal defects (ASDs) with deficient margins. When margins are deficient, a precariously placed device can appear to be held in good position by the rigid delivery cable. Once the cable is unscrewed, the device adopts the natural lie of the interatrial septum. This can occasionally expose the inadequately captured margins and lead to device embolization. Most embolizations occur immediately after release. Retrieval of the embolized device required prolonged fluoroscopy and sometimes open heart surgery. Objective : To evolve a new strategy of retrieval of a malpositioned device after unscrewing the cable before impending embolization. Materials and Methods : After deploying the device in place, a snare is passed through the delivery sheath around the cable to grip the screw on the right atrial disc of the device. With the snare holding the screw end, the device is released by unscrewing the cable. The device position is reconfirmed on echocardiography. The snare is subsequently removed if the device was stable. In case of device migration, the same snare is used to retrieve the device before it embolizes completely. Results : Snare assistance was used in 24 patients considered as high-risk for device embolization. Its usefulness was demonstrated in two patients with deficient posterior margin and small inferior margin where the device got malpositioned immediately after release. As the snare was still holding on to the screw end, the device could be retrieved into the sheath easily. Conclusion : This novel snare assisted device release strategy safeguards against device embolization in large ASDs with deficient rims and allows simplified retrieval.

Background: Tetralogy of Fallot (TOF) with subarterial ventricular septal defect (VSD) is more common among Asians than Caucasians. Compared with the regular subaortic VSD postoperative right ventricular outflow obstruction is more common because of the sub-pulmonary extension of the defect. The objective of this study is to analyze the surgical implications and outcomes of patients with TOF - subarterial VSD in the current era. Patients and Methods: In all, 539 consecutive operated patients with TOF from May 2005 to September 2012 were retrospectively reviewed. Eighty-five patients had subarterial VSD. Seventy-nine of these underwent intracardiac repair. Preoperative clinical, echocardiographic features, operative and postoperative variables were assessed. Results: The median age at surgery was 6 years and the median weight was 14 kilograms. The male to female ratio was 1.7:1. TOF with subarterial VSD was associated with frequent use of transannular patch (74.6%). The early mortality was 2.5%. Follow up was 92% complete with a mean duration of 20 months with actuarial survival of 97.3% at 5 years. Two patients required reoperation for significant right ventricular outflow tract obstruction (RVOTO) at one year and three years, respectively. Conclusions: Intra cardiac repair for TOF with subarterial VSD has low perioperative mortality and morbidity. Transannular patch augmentation of the right ventricular outflow tract (RVOT) is required in a significant proportion of these patients. Precise suturing of the VSD patch, adequate infundibular resection and lower threshold for a transannular patch placement ensures a smooth early postoperative recovery.

A wide atrial communication is important to maintain hemodynamics in certain forms of congenital and acquired heart defects. In comparison to balloon septostomy or blade septostomy, atrial septal stenting provides a controlled, predictable, and long-lasting atrial communication. It often needs a prior Brockenbrough needle septal puncture to obtain a stable stent position. A stent deployed across a previously dilated and stretched oval foramen or tunnel form of oval foramen carries higher risk of embolization. This review provides technical tips to achieve a safe atrial septal stenting. Even though this is a "How to do it article," an initial discussion about the indications for atrial septal stenting is vital as the resultant size of the atrial septal communication should be tailored for each indication.